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At least 19 recordsLinked to original sources
PubMed2004

Diagnosis and treatment of VIPoma in China: (case report and 31 cases review) diagnosis and treatment of VIPoma.

OBJECTIVES: To discuss the experience of diagnosis and treatment of VIPoma by summarizing clinical information of 31 patients with VIPoma in China. METHODS: To analyze clinical manifestations, laboratory examinations, imaging features, operation, pathologic findings, and follow-up survey of 31 patients, among them 1 case was from our hospital and the other 30 cases were reported in Chinese literature from 1977 to 2002. RESULTS: Periodical backache, skin rash, and polyps of colon were never reported previously, all of which except polyps disappeared after operation. Partial resection of superior mesenteric vein was also never reported. The mean serum value of VIP which had been very high before operation, decreased markedly after the operation (963.2 pg/ml Versus 131.9 pg/ml),the immunohistochemical expression of many kinds of digestive hormone including VIP presented positive. Hepatic involvement was 29.0%,nodal metastasis was 9.7%. CONCLUSIONS: VIPoma is rare. Typical symptoms and the serum value of VIP are keys to diagnosis before operation, Surgical resection is the most effective means for cure. surgical debulking of the tumor, somatostatin analogues can be used for good palliation.

China↗
PubMed1996

Pathophysiology and management of VIPoma: a case study.

PURPOSE/OBJECTIVES: To review the pathophysiology and clinical treatment of VIPomas, neuroendocrine tumors that secrete vasoactive intestinal polypeptide, and VIPoma syndrome. DATA SOURCES: Published clinical treatments and case studies in the medical literature and case study data from a patient's medical record. DATA SYNTHESIS: VIPomas are rare neuroendocrine tumors that cause a syndrome of life-threatening symptoms. Clinical management is complex, yet little information is available in the medical and nursing literature to guide the clinician. CONCLUSIONS: Information about the pathophysiology and management of the disease may facilitate care of these patients. IMPLICATIONS FOR NURSING PRACTICE: A case study presentation provides an example of the care required by one patient with VIPoma. The nurse has an important role in assessing, educating, and caring for the patient with VIPoma. Interventions include managing complex fluid and electrolyte imbalances, chemotherapy administration and management of side effects, activity and rest alterations, safety issues, altered social roles, and educational needs regarding medications, central lines, and follow-up care.

Adult↗
PubMed1991

Giant metastatic VIPoma in the liver.

A case of giant metastatic liver VIPoma manifesting WDHA syndrome is described. The patient was a 33-year-old Japanese male who was admitted because of persisting watery diarrhea and weight loss. Laboratory examinations revealed marked hypokalemia, mild hypercalcemia and highly elevated plasma vasoactive intestinal peptide (VIP) and glucagon levels. Intensive imaging examinations disclosed two large tumors in the liver but not in other organs, including the pancreas. Laparoscopy disclosed a slightly elevated purplish-colored large tumor on the surface of the apparently normal liver. At operation, however, a small hard nodule was observed in the tail of the pancreas. Histologically, it was revealed to be a VIPoma resembling a carcinoid or an islet cell tumor, and was identical to the liver tumors. Thus, this neoplasm was considered to be a VIPoma of pancreatic origin, with metastatic lesions in the liver.

Adult↗
PubMed1993

Calcitonin, as SMS 201-995, ameliorates the VIPoma syndrome.

A 72-year-old woman was referred to our hospital for diarrhea, abdominal and back pain, weight loss, low serum potassium level. Pathological findings and high circulating Vasoactive Intestinal Peptide (VIP) levels allowed us to diagnose "VIPoma syndrome". The patient underwent a treatment with SMS 201-995 with improvement of symptomatology and reduction of circulating VIP levels. After a wash-out period the patient was treated with salmon calcitonin with a net improvement of diarrhea, normalization of potassium serum level and reduction of VIP level. The symptomatology recurred after calcitonin withdrawal. These data confirm the effectiveness of SMS 201-995 in the VIPoma syndrome and suggest that calcitonin may ameliorate symptomatology and reduce circulating VIP level in patients with VIPoma tumor.

Aged↗
PubMed1984

Chronic diarrhea due to VIPoma in two children.

The diagnosis of vasoactive intestinal poly-peptide-secreting tumor (VIPoma) was established in two boys on the basis of watery diarrhea with hypokalemia, elevated plasma levels of vasoactive intestinal polypeptide (VIP) (range of 55-94 pmol/L), and presence of a tumor of the left adrenal gland. Despite celiac angiography, VIP estimation in blood samples taken from different parts of the body, and exploratory laparotomy, localization of the tumor in one child in vivo was unsuccessful. In the other boy, computed tomography revealed a large tumor in the left adrenal gland. Following the removal of the tumor, diarrhea ceased, and 10 days after surgery, the plasma level of VIP was 5 pmol/L. Histologically, the tumors in the two boys were found to be ganglioneuromas. The diagnosis of VIPoma is simple but localization can be very difficult. Surgical removal of VIPoma is often rewarding.

Adenoma, Islet Cell↗
PubMed2000

Metastatic jejunal VIPoma: beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil.

The VIPoma syndrome is rare. It is usually caused by a neuroendocrine tumor located in the pancreas. Somatostatin analogs and interferon-a can be helpful in the symptomatic control of the disease, but the efficacy of chemotherapy in metastatic disease is limited. We report the case of a 32-yr-old patient who had a primary intestinal VIPoma with peritoneal carcinomatosis and hepatic metastases. Somatostatin analogs and conventional chemotherapy regimens were not effective on VIPoma syndrome and tumor progression. The combination of 5- fluorouracil and interferon-alpha was associated with a major clinical improvement and tumor regression. Further investigations should evaluate the place of such a combination as a first line treatment for patients with metastatic neuroendocrine tumors.

Adult↗
PubMed2002

Vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases: dramatic and durable symptomatic benefit from hepatic artery embolization, a case report.

Neuroendocrine tumors often manifest an excess production of hormones that create severe metabolic abnormalities resulting in significant patient morbidity, independent of the tumor burden itself. VIPomas are rare neuroendocrine tumors arising from the pancreas and are associated with secretory diarrhea and electrolyte disturbances. We present a patient with VIPoma and hepatic metastases who had greater than 10 loose stools a day for 4 yr since diagnosis, despite debulking surgery, multiple antidiarrheal medications, large doses of octreotide, and targeted radioisotope injections. The patient required several hospitalizations for treatment of dehydration and electrolyte disturbances, despite receiving daily intravenous fluids at home. Hepatic artery embolization (HAE) immediately stopped the patient's diarrhea and provided a return to normal formed stools without any other symptom-support measures. One year after HAE, the patient remains asymptomatic and has returned to a productive life. HAE can be a very effective and durable treatment modality for patients with metastatic VIPomas (or other neuroendocrine tumors) and who are clinically symptomatic from the effects of hormone hypersecretion.

Antidiarrheals↗
PubMed1991

Vipoma of the pancreas complicating ulcerative colitis.

We report a case of vipoma of the pancreas in conjunction with ulcerative colitis in a 20-yr-old woman. Twenty months after the onset of ulcerative colitis, the patient complained of watery diarrhea and was found to have an electrolyte disorder. A pancreatic tumor was detected by ultrasonography and computed tomography, along with an elevation of serum vasoactive intestinal polypeptide (VIP). The secretory diarrhea diminished dramatically, and the serum VIP level decreased into the normal range immediately after resection of the pancreatic tumor. Immunohistochemical staining revealed a pancreatic vipoma. Despite removal of the vipoma, she underwent restorative proctocolectomy and ileal J-pouch anal anastomosis 2 yr later due to progression of the ulcerative colitis. Postoperative course was uneventful, with excellent functional results. Knowledge of this rare condition accompanying ulcerative colitis may help in the management of the patient with an atypical clinical course.

Adult↗
PubMed1989

Medical therapy of VIPomas.

VIP-secreting tumors are rare, but they produce a dramatic clinical picture, the most prominent feature of which is profuse, watery diarrhea and hypokalemia. A definitive diagnosis is aided by the determination of plasma VIP concentrations through the use of the sensitive radioimmunoassays that are now available. Intestinal secretion resulting from the direct action of VIP on the intestinal epithelial cell receptors accounts for the loss of fluid and electrolytes in patients with VIPoma. The hypokalemia is the result of passive and VIP-induced active secretion of potassium by colonic epithelial cells. Surgery is the most definitive treatment of VIPoma; pharmacotherapy is extremely important in controlling symptoms and stabilizing the patient prior to surgery. Sandostatin and glucocorticoids are well established agents in the management of the secretory diarrhea; other pharmacologic agents, including clonidine, indomethacin, phenothiazines, lithium carbonate, and propranolol, may be helpful in selected patients but require further study. The most potent and promising drug for the treatment of VIPoma is a new peptidomimetic agent--Sandostatin. This metabolically stable synthetic analogue of somatostatin appears, in part, to inhibit the release of VIP from the tumor and the secretion of chloride by the intestine. In addition to controlling the diarrhea, it may have a direct effect on the tumor in reducing its size.

Adenoma, Islet Cell↗
PubMed1994

In vitro release of vasoactive intestinal polypeptide and pancreatic polypeptide from human VIPoma cells and its inhibition by somatostatin analogue (SMS 201-995).

BACKGROUND: The purpose of the present study was to determine whether vasoactive intestinal polypeptide (VIP) is released from the tumor cells of VIPoma and if so then to attempt to show how its release is regulated by cultured human VIPoma cells. METHODS: A resected specimen of a pancreatic tumor from a patient with watery diarrhea, hypokalemia, and achrohydria syndrome was examined. The dissociated cells were obtained by collagenase digestion of the tumor tissue and were cultured in vitro. RESULTS: The extraction of tumor cells disclosed that the cells contained VIP and pancreatic polypeptide (PP). Neither insulin, glucagon, somatostatin nor pancreastatin was detected. Immunohistochemically, 40% to 60% of the cells in the tumor stained positively for VIP and 1% to 5% stained positively for PP. The dissociated cells became reaggregated in the culture (50 to 300 microns) and could be maintained in vitro. Incubation experiments revealed a simultaneous in vitro release of VIP and PP with a significant increase by either carbachol or phorbol myristate acetate but not by theophylline or caerulein. Atropine completely abolished the stimulatory effects of carbachol on VIP and PP release. Octreotide (somatostatin analogue [SMS 201-995]) significantly inhibited the carbachol and phorbol myristate acetate-stimulated VIP and PP release. CONCLUSIONS: These findings show the in vitro release of VIP and PP from the VIPoma cells and also provide evidence for the direct inhibitory effect of somatostatin analogue on both the VIP and PP release from the tumor cells.

Adult↗
PubMedDate not supplied

Liver VIPoma: report of two cases and literature review.

Two cases of neuroendocrine tumor in the liver, positive for VIP, without evidence of a primary tumor outside the liver is presented. One patient had a VIPoma syndrome with diarrhea, hypokalemia, and hypercalcemia, all symptoms were reversible after treatment consisting of somatostatin analogue and arterial liver embolization followed by liver resection. The other patient showed no endocrine symptoms. To the best of our knowledge, VIPomas apparently primary in the liver have not been previously described.

Adult↗
PubMed1995

Pancreatic vipomas: spectrum of presentation and evolution of diagnostic and therapeutic modalities.

Two patients are reported who presented with symptoms characteristic of a pancreatic vipoma. The necessity to measure more than one plasma VIP level for diagnosis, and the delay between the onset of illness and diagnosis is illustrated by both cases. Evidence suggests that vipomas are still under reported. The evolution of sophisticated diagnostic and therapeutic modalities over the twenty-five years separating both presentations is discussed.

Combined Modality Therapy↗
PubMed1983

Diarrhoea in vipoma patients associated with cosecretion of a second active peptide (peptide histidine isoleucine) explained by single coding gene.

Peptide histidine isoleucine (PHI), first isolated from pig intestine, is distributed identically to vasoactive intestinal peptide (VIP) in all mammals. 42 patients with high plasma VIP secondary to VIPoma also had very high plasma PHI-like immunoreactivity, in a constant ratio to VIP. None of 125 patients with other endocrine tumours had high levels of either peptide. VIPoma tissue from 20 patients also contained PHI shown by immunocytochemistry to be produced by the same cell as VIP. Messenger RNA(mRNA) from one of these tumours contained the codes for VIP and a separate PHI-like sequence. Human PHI-like sequence differed from porcine PHI in only two aminoacid residues. A single cell thus produces two separate regulatory peptides with apparently similar potencies but different spectra of activity. In normal tissue the constant coproduction of two active neuropeptides by a single neuron provides further evidence against the doctrine of one neuron producing only one neurotransmitter.

Adenoma, Islet Cell↗
PubMed1998

The cost-effectiveness of octreotide acetate in the treatment of carcinoid syndrome and VIPoma.

Markov modeling was used to evaluate the cost-effectiveness of octreotide in treating carcinoid syndrome and VIPoma. For each condition, using octreotide was associated with doubled survival time. Octreotide was cost-effective for treating carcinoid tumor ($752 per additional year of life, two additional years on average), and cost saving for VIPoma.

Antineoplastic Agents, Hormonal↗
PubMed2002

[Sonographic detection of a "VIPoma" in a small child with intractable gastroenteritis].

As case report we describe a rare cause of intractable "gastroenteritis" detected by ultrasonography. The 14 months-old boy was admitted to hospital because of intensive dehydration due to massive vomiting and diarrhoea. A salmonella enteritis with intractable hyponatraemia and hypokalaemia was thought to be the cause. After a dramatic relapse during oral treatment measures, further extensive laboratory tests finally disclosed an elevated serum level of vasoactive intestinal polypeptide ("VIP"). The VIP secreting tumor ("VIPoma") was detected ultrasonographically in a retroperitoneal localization mediocaudally of the right kidney. Diffuse distinct calcifications and an increased perfusion could be demonstrated. Intraspinal tumour spread was excluded by magnetic resonance imaging. After complete surgical removal of the tumour the clinical symptomatology normalized promptly and permanently. A VIP-excreting ganglioneuroblastoma with low grade growth fraction ("VIPoma") was diagnosed histologically. Common gastroenteritis in childhood represents no indication for ultrasound. In cases of unclear and therapy-resistant symptomatology, however, diagnostic work-up should include ultrasonography to search for retroperitoneal or pancreatic VIP-excreting tumours.

Gastroenteritis↗
PubMed1984

[Radiodiagnosis of glucagonoma and vipoma, 2 rare endocrine pancreatic tumors].

From the non-B-cell tumors of the pancreas the gastrinoma (Zollinger-Ellison-syndrome) is the best known. The glucagonoma and the vipoma are less known and less common. The diagnosis of these syndroms, produced by overproduction of hormones, is a clinical one. The radiodiagnost has to localize the primary tumor and to exclude metastases. The x-ray findings of the glucagonoma in 3 patients and of the vipoma in 1 patient are described.

Adenoma, Islet Cell↗
PubMed1995

[Metastasizing pancreatic vipoma. Its diagnosis and therapy with the somatostatin analog octreotide].

HISTORY: An 82-year-old woman was hospitalized for anaemia of 4.8 g/dl after having suffered for about one year from watery treatment-resistant diarrhoea, causing a weight loss of ca. 10 kg. FINDINGS: Computed tomography, magnetic resonance imaging and endosonography revealed a 2.5 x 2.0 cm space-occupying mass in the body of the pancreas. Taking into account electrolyte abnormalities (potassium 2.7 mmol/l), marked metabolic acidosis (pH 7.16, base excess -20.3 mmol/l) and achlorhydria, an increased serum concentration of vasoactive peptide (VIP) of 548.5 pmol/l confirmed a VIPoma. Somatostatin-receptor scintigraphy also demonstrated a metastasis, 1.8 cm in diameter, in the region of the right ovary. TREATMENT AND COURSE: Under administration of somatostatin analogue octreotide (150 micrograms three times daily subcutaneously) the symptoms quickly disappeared and the stools as well as electrolyte and acid-base balances became normalized. After 5 months of treatment the levels of VIP, pancreatic enzymes and gastrin were within normal limits or clearly suppressed. There has been no recent evidence of tumour progression. CONCLUSION: This case demonstrates so far successful suppression of a metastasizing VIPoma with the somatostatin analogue octreotide, the metastasis having been revealed first by somatostatin-receptor scintigraphy.

Aged↗
PubMed1996

Massive amyloid deposition in pancreatic vipoma: a case report.

We present a case of a pancreatic islet cell tumor, demonstrating typical clinical and immunohistochemical characteristics of a VIPoma, but with the novel feature of massive stromal amyloid deposition. Amyloid deposition has been reported in association with insulinomas of the pancreas, but as far as the available literature suggests, it has not been reported in VIPomas. Islet associated polypeptide (IAPP) and calcitonin are both candidates for the amyloidogenic peptide in this case, based on observations in other tumors.

Aged↗